Mitochondria (from the Greek, meaning “string grain” — yeah, it’s lame, but it sounds good in Greek) are independent little one-celled organisms that live inside your cells and make energy for them. If you ever studied the ATP cycle (also called the Krebbs cycle or the citric acid cycle, depending on where you went to school and how deeply they went into it), then you should know that this is where the ATP cycle takes place.

Without mitochondria, you have no way of converting food into energy.

When you were being conceived, half your cells’ genes came from your mother and half from your father. All of the other stuff that goes inside a cell came from your mother. This includes the mitochondria. (This is why mitochondrial DNA is used to track maternal inheritance: it always comes down the female line.) Your mother’s cell hosts conception, just as (normally) your mother’s body hosts gestation.

Mitochondria have a fairly smooth outer layer and a deeply-rumpled inner layer. Most of the action happens inside the rumpled layer. This is where the ribosomes, most of the fluids and loose protein, and the ATP-making particles hang out.

Cells, including mitochondria, need various proteins to do their work with. Large proteins get carefully handed from the outside world, through the outer layer of the mitochondrion (singular of “mitochondria” — sorry, it’s still Greek), then into the inner layer.

If the smooth outer layer is damaged, this makes this transfer process screw up, and the inner layer gets disrupted, ripping up the cell. Granules and nucleic acids all over the place. Bang goes that ATP production.

Knowing why it’s so damnably exhausting to walk a mile, when it used to be fun — fun! — to run 3, is a bit of a relief. First question that leaps to my mind: How do I fix ’em? How do I give them what they need to get better and protect themselves? The answer seems simple: antioxidants are what’s needed to prevent and repair that damage (good explanation of that here) to the walls of the mitochondrial cell. Mitochondria are both the biggest makers of reactive oxygen species and the biggest scavengers of them, so of course it makes sense that that’s exactly the kind of help they need when they can’t keep up.

Downing antioxidants by the bucketful is one way to get them in. Intriguing for three reasons:

Kind of depressing for one simple reason: it’s iffy whether, once you’ve got the disease process going, the antioxidants can get where they’re needed and save your poor beleaguered mitochondria. … Having said that, I notice that the writers of that article seem to be trying to sell something, and that makes me very suspicious of their conclusions.

Next, I’ll offer suggestions for patients, suggestions for clinicians, and then wind this up with a foray into the question of whether mitochondrial issues have a genetic component, like being X-linked — the way a cat’s fur color is!

For people with CRPS — So what is a poor, confused CRPSer to do?

Two things that you hardly need reminding of:

Trust your sense of your own body.

Do what works for you.

Most antioxidants are not going to hurt you, without letting you know first (that is, make you nauseous or feel funny.) Take vitamin C in doses no larger than 500mg, since larger doses tend to trigger your gut to throw the C away. Go ahead and try stress-vitamins, co-enzyme Q-10, N-acetylcysteine, hair-skin-&-nails vitamins (these are really fat-soluble antioxidants) … try things, take what helps, and put aside the rest if they don’t do anything. Keep in mind that things change: what doesn’t work now might work later, and vice-versa.

For antioxidant powerhouses, look for dark-red and dark-blue fruits: pomegranates, blueberries, red wine, chocolate (though some CRPS people have to avoid that for its nerve effects), mangosteen (my favorite fruit), cranberries, and so on.

Stay smart. Stay loose. Keep going.

For medical people — clinical takeaways:

Most treatment standards, particularly for CRPS, are based on science that’s over a decade old. They shouldn’t be changed blithely but they can certainly be improved. There is plenty of room for that.

The following points are intended as additions to the standards you follow for CRPS, as they are good guidelines for mitochondrial and neurologic support in a system compromised by CRPS.

After any limb surgery, give Vitamin C 500 mg, QD or BID, for a couple weeks beforehand and 30-50 days after — or to metabolic tolerance, if that’s too much. Use a food-associated form for best uptake. This one intervention will reduce the risk of developing CRPS by 80%, according to the best current data.

We assume your patients are taking an adequate multivitamin and are eating plenty of greens, dark fruits, and wholesome proteins. So make sure they are. Direct them to food bank, food stamps or other food assistance as needed. Give recipes. (No kidding.) 2 benefits: better antioxidant uptake if taken with antioxidant-rich food, and increasing the patient’s own sense of agency/participation improves pain and affect. (If you don’t believe in multivitamins, then get out of the supermarket/pharmacy and get some real ones.)

Give “uber-antioxidants” like ubiquinone (co-Q 10), N-acetylcysteine, or glutathione. There are indications that these can provide substantial benefit — though again, not normally curative of chronic CRPS. They are impressive, especially for mitochondrial-dysfunction issues.

These ranges are empirical; if you can find the funding to do the science to develop more reliable ranges for this population, so much the better.

Adequate tissue oxygenation and perfusion can return substantial function and significantly reduce pharmacologic burden. Patients can demonstrate this, even where the data have not been published and peer reviewed. Therefore, use antioxidants rigorously and intelligently.

Why all that anti-oxidation when the medical literature is not definitive? 2 reasons, which you ought to know for yourselves:

Between the cortisol and systemic oxidative stresses, it can’t hurt and it will help something. You’ll see a distinct improvement in affect, activity, motivation and well-being when the dose is optimized, even if it can’t be expected to be curative. Making your patient’s life more bearable is an essential part of your job.

Let’s say this together, everyone: statistics mean nothing in the case of the individual. Accepted, standardized medicine is what you start with, but, when your case is taking you out to the margins, you go to the margins, because that’s where your success is most likely to await.

Keep in mind that doctors are not the only scientists interested in the human body. Be prepared to look into other disciplines for leads when your own offers no good options.

The accepted style is very different, but the info they have is tremendous.

Forward-looking thoughts:

Consider infusing vitamin K into CRPS-damaged tissues. I would love to see studies on that.

Figure out how to deliver antioxidants in a targeted way. (Now! Please!) This would be a good way to save a lot of lives and end tons of misery.

… And for all curious people …

Let’s go back to mitochondria in reproduction. Kind of in an X-rated way, figuratively speaking.

We know that women have two X chromosomes. The Y chromosome is a stubby little object with hardly any data to use, unless you’re into color-blindness or hemophilia; this means women have quantities of extra data, which can have even more devastating effects (as in, Down syndrome.) So how to handle the extra genes?

Pick one. Simple as that.

Shortly after conception, when the cells are just dividing like mad and haven’t decided what to be yet, every single cell turns off one of its two X chromosomes; each of that cell’s daughter cells inactivates the same X chromosome. As the cells continue to multiply, then fill out, fold, bend around, and specialize, to become a whole, separate being, it means that X-linked traits appear in a mottled pattern throughout the body, as the two sets of daughter cells continue reproducing and passing on their particular X-activations. Isn’t that curious?

As an especially decorative instance, cats’ hair color is an X-linked trait:

Cool, huh? Love her accent, too.

But this fact brings me to a serious question about mitochondrial disease. If mitochondria are sex-linked, is there a relationship between the X chromosome and mitochondrial expression? It seems improbable that there wouldn’t be, because mitochondria reside inside the cell, and the cell’s action is determined by the genes within it. The mitochondria had to have developed a special relationship with the X’s in the 23rd chromosomal pair, after all those millenia.

It’s generally accepted that mitochondrial diseases are due to toxification or to complex, multigenetic issues. Ok, fine. But what about mitochondrial vulnerabilities that don’t become pathologic until they are damaged in some other way? To what degree is toxification an issue related to X-activation? In other words, is mitochnodrial vulnerability related to vulnerabilities in the active X chromosome?

Is there a patchy characteristic to the early stages of mitochondrial destruction? — You know, the early stages of rare disorders, the time when it’s impossible to get a diagnosis because the doctors are all so busy chasing their own tails around your irrational symptoms and their own ignorance.

Is that initial “mottled” activity one reason why these diseases are so damn weird?

I originally created this blog as a place to digest articles on medicine and biomedicine — especially as they relate to real, live human beings of the kind who need to use medicine and biomedicine. After all, needing it means our systems are not quite normal.

I have a condition that punches holes in my memory and cognition. This means that, even though this biomedical stuff is meat and drink to me, I have to look up things that — with my old brain — I used to know like the back of my hand. (That is, the hand where the CRPS started, naturally.)

Really basic things, like the names of our handful of neurotransmitters, each with its many jobs; or the role of the pituitary gland and its intense relationship with … well, with every other regulatory part of the body.

So I’ll post a couple of tutorials on these subjects here, for both you and me to refer to at need. If I’m really clever, I’ll post them as pages which you can access easily; for now, I’ll be happy to get them up at all.

I originally created this blog as a place to digest articles on medicine and biomedicine — especially as they relate to real, live human beings of the kind who need to use medicine and biomedicine. After all, needing it means our systems are not quite normal.

I have a condition that punches holes in my memory and cognition. This means that, even though this biomedical stuff is meat and drink to me, I have to look up things that — with my old brain — I used to know like the back of my hand. (That is, the hand where the CRPS started, naturally.)

Really basic things, like the handful of neurotransmitters each with its many jobs, or the role of the pituitary gland and its intense relationship with … well, with every other regulatory part of the body.

So I’ll post a couple of tutorials on these subjects here, for both you and me to refer to at need. If I’m really clever, I’ll post them as pages which you can access easily; for now, I’ll be happy to get them up at all. Soooo ….

One of the most expensive and brutal treatments I’ve tried was the SCS (spinal cord stimulator) trial. This involved hammering two metal-laced widgets the size of drinking straws into that tiny, sensitive space entirely surrounded by bone which we call the spinal cavity. The one that’s usually fully-filled by a mass of busy, sensitive nerve tissue.

My arms hadn’t hurt so little in years. My spine, back, neck and head had never hurt so much in my entire freaking _life_. At the mere rememberance, I still feel the shoulder, scapular and upper-back muscles twist themselves into that ghastly position that caused the least pressure against my cord. My neck is going to be cramping the rest of the day. Wish I hadn’t mentioned it.

So yeah, that was bad. I wrote Medtronics a bunch of notes from the nurse/patient/biogeek/engineer perspective. I have seen no indication that they did anything but circular-file them, so I feel free to discuss it now. They’ve had their grace period.

There’s no reason for such flat-out stupid, ham-handed, ignorant engineering. I’ve tried to see an upside to the current SCS tech, and I just can’t.

Welcome to the future of implantable devices — especially those going into immunoreactive, sensitized systems:

Meet the carbon nanotube: body-friendly, weavable, conductive, cheap, easy to work with. Does its job on, easily, one thousandth the scale of silicone and wire.

Why is this on my mind? (Gentlemen, look away and hum.) First day of my cycle after a month of progress in rebuilding basic endocrine responses. EVERYTHING is supersensitive and hyperreactive. I want a carbon nanotube *body!*

Best I can do is coconut water. It really smooths out the bumps, if I drink enough of it. Here’s to 21st century tech, and hoping it makes it into something as profitable as biomedicine!

The authors did an autopsy on one person with longstanding CRPS and did comparative autopsies on 4 people who did not have CRPS. They checked samples from the neck, thorax, and low-back for microglia and astrocytes. These are the kinds of cells that not only are part of the nervous system’s immune response, but also increase the transmission of pain signals. That means, inflammation plus more pain! They found plenty in the CRPS patient’s spine.

They also found that the normal cells in the dorsal horn of the spine — the ones that carry sensations of light touch, vibration, and proprioception (the sense of the body in space) — are significantly fewer in the CRPS patient. This makes sense of the fact that allodynia (light touch) gets worse, vibration is so agonizing (making both riding public transit and holding a steering wheel pretty horrible), and we get clumsy over time because we can’t quite feel where our bodies are in space.

These strange cellular changes were found “most prominently at the level of the original injury, but extending throughout the entire length of the spinal cord.” That means that the allodynia, diminished balance, etc. physically spread from the original dorsal root, all the way up and down the spine, affecting the whole physical self.

So, with more cells for pain and immune attack, and fewer cells to transport normal messages of light touch, vibration, and proprioception, we have some stunningly clear evidence that the spreading allodynia, clumsiness, and intolerance to vibration is NOT IMAGINARY.

Given how many people get told that it’s all in their heads, they’re hurting because they’re thinking wrong or because they were abused as children, etc., this is an important thing to keep in mind. Let’s keep the cart behind the horse.

I have CRPS-1/RSD/causalgia, and when your condition has more than one name, it’s a bad sign. I was a nurse, I was a tech writer, and I remain fascinated by health and technology.

Some parts of my brain have blown gaskets, but examining the science relating to neurology/immunology/endocrinology — and mulling how it could work in real life — seems to go just fine. It’s appropriate to both my professions that I want to track, document, and share what I learn.

You’re invited to watch and engage in this interesting journey. It’s taking place at an unimaginably rich, burgeoning age of technological development and biological understanding.